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Nephrotic syndrome is an unusual manifestation of IgA Nephropathy (IgAN). Some cases respond to steroid treatment. Here we describe a case-series of IgAN patients with steroid-responsive nephrotic syndrome. Twelve patients with IgAN with steroid-responsive nephrotic syndrome were evaluated and followed up. All patients presented with generalized edema. Renal insufficiency was found in two patients. The renal biopsy of eight patients revealed wide foot process effacement in addition to the typical features of IgAN. They showed complete remission after steroid therapy. Seven relapses were reported in five patients; six of the relapsed cases responded to steroid therapy. Compared with steroid-non-responsive patients, the patients with steroid-responsive nephrotic syndrome had shorter symptom duration, more weight gain, more proteinuria, and lower histologic grade than did those that had steroid-non-responsive nephrotic syndrome at presentation. None of the responders progressed to end stage renal disease, whereas five (38%) non-responders required dialysis or renal transplantation. Patients with IgAN who have steroid-responsive nephrotic syndrome likely have both minimal change disease and IgAN. The clinical features of sudden onset of generalized edema, initial heavy proteinuria and initial severe hypoalbuminemia might help identify the subset of patients, especially in low grade IgAN.

Background/Aims: Hypertension is one of the most significant prognostic factors of immunoglobulin A nephropathy (IgAN). We investigated the role of polymorphisms of hypertension-related genes in the clinical impact of IgAN. Methods: A total of 238 IgAN and 300 healthy cohorts were studied. The polymorphisms of angiotensinogen (AGT M235T), the angiotensin II type 1 receptor (A1166C), aldosterone synthase (C-344T), α-adducin (G460W) and endothelin-1 (K198N and 3A/4A) were determined. Results: The genotype distributions of the polymorphisms were similar between patients and controls. The individual genotypes taken alone were not associated with the development of hypertension or progression of renal dysfunction. Although AGT M235T was not associated with the development of hypertension in either sex, men with M235T TT were found to be at an increased risk of IgAN progression compared to those with the other genotypes (p = 0.019). In the Cox regression model with adjustment for clinical risk factors, including age at diagnosis, hypertension, serum creatinine and urinary protein excretion at renal biopsy, AGT M235T TT variant was an independent risk factor only for male patients (hazard ratio 5.848; p = 0.005). Conclusion: Our results suggest that the AGT M235T polymorphism is associated with the progression of IgAN in Korean male patients.

A successful transplantation, across a positive crossmatch barrier, is one of the most persistent long-standing problems in the field of kidney transplant medicine. The aim of this study was to describe seven consecutive living renal transplantations in recipients with positive crossmatch for donors or positive for donor specific antibodies (DSAs). A preconditioning regimen including plasmapheresis and intravenous immunoglobulin was delivered three times a week until the crossmatch and/or DSAs became negative. Mycophenolate mofetil and tacrolimus were started two days before the plasmapheresis. The protocol was modified to include administration of anti-CD 20 antibody (rituximab, 375 mg/m2) from the patient number 3 through the patient number 7. All seven patients achieved negative conversion of the crossmatch or DSAs, and the kidney transplantations were successfully performed in all cases. Acute cellular rejection occurred in two patients, which were subclinical and controlled with high dose steroid treatment. Antibody-mediated rejection occurred in one patient, which was easily reversed with plasmapheresis. All recipients attained normal graft function during the 7-24 months of follow up. Our study suggests that sensitized patients can be transplanted successfully with desensitization pretreatment.

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